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Thrombotic thrombocytopenic purpura (TTP) is a disease characterized by small blood clots
throughout the body that can damage major organs and cause death. TTP is treated with plasma
exchange (also called "plasmapheresis"). Patients who do not respond initially to plasma
exchange often are helped by later treatment with rituximab. The purpose of this study is to
see whether combining low doses of rituximab with plasma exchange will help patients get
better sooner and reduce the chance of getting TTP again.

This is a pilot safety/efficacy study of adjuvant low dose rituximab (100 mg/week x 4 doses)
plus standard plasma exchange and corticosteroids for the treatment of thrombotic
thrombocytopenic purpura (TTP) with severe ADAMTS13 deficiency. Results for study subjects
will be compared to historical controls treated initially with plasma exchange and
corticosteroids. This study proposes to test the hypothesis that adjuvant low dose rituximab
may decrease the incidence of a composite primary endpoint (exacerbations or refractory
disease) in acquired TTP with severe ADAMTS13 deficiency. A novel ADAMTS13 assay will be used
to identify patients with TTP and severe ADAMTS13 deficiency for enrollment, and to assess
the utility of ADAMST13 as a biomarker for response to therapy and prognosis.